Case Report

Are Normal coronary arteries a typical feature of apical ballooning syndrome?

Abstract

Apical ballooning syndrome (ABS) is a new and uncommon, yet very interesting, clinical phenomenon regarded as one of the important elements of differential diagnosis in acute myocardial infarction. It was first described in 1990. The absence of obstructive coronary artery disease among others is a typical feature of ABS, required to make a final diagnosis. We describe a case of a woman with ultrasonographically confirmed tight stenosis in the right coronary artery, yet showing all other characteristics of ABS.

In the past decade, the widespread availability of invasive diagnostics for acute coronary syndromes (ACSs) has led to identifying a special subgroup of ACSs preceded by emotional or physical stress. First described by Japanese researchers in 1990, this relatively new clinical entity known under various names, such as apical ballooning syndrome (ABS), Tako-tsubo, or stress-induced or ampulla cardiomyo- pathy, is currently increasingly reported and accounting for as high as 1% to 2% of all cases with preliminary diagnosis of myocardial infarction [1,2]. The latest standard definition of ABS is composed of the following 4 criteria: (1) transient hypokinesis, akinesis, or dyskinesis of the left ventricular (LV) midsegments with or without apical involvement; (2) absence of obstructive coronary artery stenoses; (3) ST-T abnormalities with/or troponin rise; and (4) absence of specific conditions such as pheochromocytoma, myocarditis, Hypertrophic cardiomyopathy, Intracranial bleeding, or recent head trauma [2,3]. To establish a clinical diagnosis, all of the above are needed. We present a case of a woman with Significant stenosis of the right coronary artery , yet showing all other features characteristic for ABS.

A 53-year-old woman with the history of hypertension, hypercholesterolemia, and cigarette smoking was transferred from a primary care unit outside the capital city to the invasive cardiology department at the university hospital, with a preliminary diagnosis of ST-segment elevation myocardial infarction. She was complaining of retrosternal continuous chest pain and dyspnea lasting for 5 hours, and the symptom onset was preceded by significant emotional stress associated with professional career. On admission, the

patient was hemodynamically stable, with a systolic blood pressure of 130 mm Hg and a regular sinus rhythm of 84 per minute. On physical examination, apart from obesity (body mass index, 32 kg/m²), no other abnormalities were noted. The 12-lead electrocardiogram recording revealed an ST-segment elevation in leads I, aVL, V2 to V6, with the highest elevation of 0.3 mV in V3 and V4 (Fig. 1A). The plasma troponin I was elevated to 6.5 ng/mL and creatine kinase-MB to 15.5 ng/mL.

Patient was transferred from the emergency department directly to the catheter laboratory. Left ventriculogram showed akinesis of LV midsegments, with normal contractility of the apical and basal parts (Fig. 2A and B). Coronary angiography revealed normal left coronary artery (Fig. 2C), but there was a short and angiographically significant lesion in the second segment of the RCA that did not change or resolve with intracoronary nitroglycerin (Fig. 2D). To fully exclude the focal spasm of the RCA, intravascular ultrasound (IVUS) was performed. This examination proved this lesion to be highly significant according to IVUS criteria, with 2.8 mm² of minimal lumen cross-sectional area and 81.5% plaque burden (Fig. 2E). The IVUS was also performed in the left anterior descending (LAD) and left circumflex arteries and revealed neither significant lesions nor any evidence of ruptured plaque. Thus, IVUS-guided successful percutaneous coron- ary intervention of RCA was performed with 3.5 x 16-mm bare metal stent implantation deployed at 16 atm and postdilated with 4.0 x 13 noncompliant balloon (Fig. 2F). Baseline standard Echocardiographic examination and after contrast injection (to enhance endocardial border definition) demonstrated left LV wall-motion abnormalities character- istic for ABS with Tako-tsubo potlike shape observed in systole (Fig. 3A-D). In the following days on aspirin, clopidogrel, ?-blocker, and statin therapy, the patient was free of chest pain and hemodynamically stable. In serial ECG recordings, evolutionary changes appeared with ST- segment elevation resolution and formation of deep T waves in V1-V3, Q waves in V1, V2, and aVL, and significant prolongation of QT interval (corrected QT was 638 milliseconds; Fig. 1B). The patient was discharged on the eighth day. Control echocardiography after 4 weeks showed complete resolution of contractile impairment in LV segments previously described as asynergic (Fig. 3E and F). Recent growing incidence of reporting has led to establishing ABS as one of the uncommon but clinically

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Fig. 1 The ECG recordings obtained on admission showing ST-elevation (A) and 72 hours later with characteristic evolutionary changes (B).

interesting syndromes. Its importance has been appreciated in the last 2007 official guidelines for the management of ACSs without ST-segment elevation, both by the American College of Cardiology/American Heart Association and European Society of Cardiology [4,5]. Current definitions of ABS vary slightly with different authors, but the presence of normal or not significantly stenosed coronary arteries is seen in each of them [6-9].

Our patient displayed most of the typical features of ABS

–her symptoms were triggered by emotional stress, and ventriculographic, electrocardiographic, and echocardio- graphic changes during the acute phase were characteristic of ABS. Moreover, only modest troponin I elevation, evolution of ST-segment elevation in the subacute phase, and complete resolution of contractile dysfunction at 1 month also strongly suggest ABS as the primary cause of symptoms. Nevertheless, according to any of the present definitions, this syndrome could not be classified as ABS because of the presence of Significant coronary lesion. In the

series of 19 cases of ABS described by Wittstein et al [10],1 patient was reported as having 70% narrowing in LAD. However, there are 3 aspects that distinguish this case from our patient. First, the lesion is still borderline; second, one could not exclude Coronary spasm because no IVUS examination of LAD was performed; and third, one could suspect especially with a long LAD supplying also the apex and midsegments of the interior wall that the primary cause for contractile and electrocardiographic changes could then be secondary to transient thrombotic occlusion of LAD not seen at the time of angiography. To the best of our knowledge, our patient is the first case of “ABS-like” syndrome, with incidental finding of ultrasonographically confirmed significant plaque in the artery supplying the territory unlikely to cause contractile dysfunction of the midsegments of the LV. The diagnosis of ABS is also strongly supported by the absence of any significant or ruptured plaque in the left coronary artery. This 1 case is probably not enough to change the definition of ABS, but we

Fig. 2 The LV ventriculography in systole (A) and diastole (B) showing akinesis of midsegments with normal function of apical and basal parts. Angiography with normal left coronary artery (C) and significant stenosis in mid-RCA (D). The IVUS examination demonstrating tight stenosis before percutaneous coronary intervention (E) and after 3.5 x 16-mm stent implantation (F).

believe it may at least cast a new light on the clinical presentation of this interesting syndrome.

Zenon Huczek MD, PhD Krzysztof J. Filipiak MD, PhD Janusz Kochman MD, PhD

Marek Roik MD Radoslaw Piatkowski MD

Grzegorz Opolski MD, PhD

1st Department of Cardiology The Medical University of Warsaw Central University Hospital

02-097 Warsaw, Poland E-mail address: zhuczek@wp.pl

doi:10.1016/j.ajem.2008.01.046

Fig. 3 Baseline echocardiograms in apical 2-chamber view without and with contrast in systole (A, C) and diastole (B, D) showing deep hypokinesis of the midsegments of LV ventricle. Complete restoration of contractile function in follow-up echo at 1 month (E, F).

References

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2. Prasad A. Apical ballooning syndrome: an important differential diagnosis of acute myocardial infarction. Circulation 2007;115:e56-9.
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